Mohsenian Samin, Seidizadeh Omid, Palla Roberta, Jazebi Mohammad, Azarkeivan Azita, Moazezi Somayeh, Baghaipour Mohammad Reza, Menegatti Marzia, Peyvandi Flora
Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy.
Iranian Comprehensive Hemophilia Care Center, Tehran, Iran.
Haemophilia. 2023 May;29(3):827-835. doi: 10.1111/hae.14773. Epub 2023 Mar 10.
The assessment of clinical history is crucial before referring a patient for further laboratory testing. Bleeding assessment tools (BAT) are developed to standardize clinical evaluation. A small number of patients with congenital fibrinogen deficiencies (CFDs) have been evaluated with these tools without definitive results.
We compared the adequacy of the ISTH-BAT and the European network of rare bleeding disorders bleeding score system (EN-RBD-BSS) to identify patients with CFDs. The correlation between the two BATs and fibrinogen levels and patient clinical grade severity was further analyzed.
We included 100 Iranian patients with CFDs. Routine coagulation and fibrinogen-specific tests (fibrinogen antigen [Fg:Ag] and activity [Fg:C]) were performed. The ISTH-BAT and EN-RBD-BSS were used to assess the bleeding score (BS) of all patients.
The ISTH-BAT and EN-RBD-BSS median (range) were 4 (0-16) and 2.21 (-1.49 to 6.71), with a statistically significant moderate correlation between the two systems (r = .597, P < .001). In patients with quantitative deficiencies (afibrinogenemia and hypofibrinogenemia), the correlation between Fg:C and the ISTH-BAT was moderately negative (r = -.4, P < .001), while the correlation between Fg:C and the EN-RBD-BSS was weakly negative (r = -.38, P < .001). Overall, 70% and 72% of patients with fibrinogen deficiencies were correctly identified by both the ISTH-BAT and EN-RBD-BSS, respectively.
These results suggest that in addition to the ISTH-BAT, the EN-RBD-BSS may also be useful in identifying CFD patients. We found a significant level of sensitivity for detecting fibrinogen deficiency in the two BATs, and bleeding severity classification correctly identified severity grades in almost two-thirds of patients.
在将患者转诊进行进一步实验室检查之前，临床病史评估至关重要。出血评估工具（BAT）旨在规范临床评估。少数先天性纤维蛋白原缺乏症（CFD）患者已使用这些工具进行评估，但结果并不明确。
我们比较了国际血栓与止血学会出血评估工具（ISTH-BAT）和欧洲罕见出血性疾病网络出血评分系统（EN-RBD-BSS）在识别CFD患者方面的适用性。进一步分析了两种BAT与纤维蛋白原水平及患者临床严重程度分级之间的相关性。
我们纳入了100例伊朗CFD患者。进行了常规凝血和纤维蛋白原特异性检测（纤维蛋白原抗原[Fg:Ag]和活性[Fg:C]）。使用ISTH-BAT和EN-RBD-BSS评估所有患者的出血评分（BS）。
ISTH-BAT和EN-RBD-BSS的中位数（范围）分别为4（0-16）和2.21（-1.49至6.71），两个系统之间存在统计学上显著的中度相关性（r = 0.597，P < 0.001）。在定量缺乏（无纤维蛋白原血症和低纤维蛋白原血症）患者中，Fg:C与ISTH-BAT之间的相关性为中度负相关（r = -0.4，P < 0.001），而Fg:C与EN-RBD-BSS之间的相关性为弱负相关（r = -0.38，P < 0.001）。总体而言，ISTH-BAT和EN-RBD-BSS分别正确识别了70%和72%的纤维蛋白原缺乏症患者。
这些结果表明，除了ISTH-BAT外，EN-RBD-BSS在识别CFD患者方面可能也有用。我们发现两种BAT在检测纤维蛋白原缺乏方面具有显著的敏感性水平，并且出血严重程度分类在几乎三分之二的患者中正确识别了严重程度等级。
